Hyper-IgG4 disease: report and characterisation of a new disease

BACKGROUND: We highlight a chronic inflammatory disease we call 'hyper-IgG4 disease', which has many synonyms depending on the organ involved, the country of origin and the year of the report. It is characterized histologically by a lymphoplasmacytic inflammation with IgG4-positive cells and exuberant fibrosis, which leaves dense fibrosis on resolution. A typical example is idiopathic retroperitoneal fibrosis, but the initial report in 2001 was of sclerosing pancreatitis. METHODS: We report an index case with fever and severe systemic disease. We have also reviewed the histology of 11 further patients with idiopathic retroperitoneal fibrosis for evidence of IgG4-expressing plasma cells, and examined a wide range of other inflammatory conditions and fibrotic diseases as organ-specific controls. We have reviewed the published literature for disease associations with idiopathic, systemic fibrosing conditions and the synonyms: pseudotumour, myofibroblastic tumour, plasma cell granuloma, systemic fibrosis, xanthofibrogranulomatosis, and multifocal fibrosclerosis. RESULTS: Histology from all 12 patients showed, to varying degrees, fibrosis, intense inflammatory cell infiltration with lymphocytes, plasma cells, scattered neutrophils, and sometimes eosinophilic aggregates, with venulitis and obliterative arteritis. The majority of lymphocytes were T cells that expressed CD8 and CD4, with scattered B-cell-rich small lymphoid follicles. In all cases, there was a significant increase in IgG4-positive plasma cells compared with controls. In two cases, biopsies before and after steroid treatment were available, and only scattered plasma cells were seen after treatment, none of them expressing IgG4. Review of the literature shows that although pathology commonly appears confined to one organ, patients can have systemic symptoms and fever. In the active period, there is an acute phase response with a high serum concentration of IgG, and during this phase, there is a rapid clinical response to glucocorticoid steroid treatment. CONCLUSION: We believe that hyper-IgG4 disease is an important condition to recognise, as the diagnosis can be readily verified and the outcome with treatment is very good

A GIS-based method for evaluating sediment storage and transport in large mining-affected river systems

Currently, the evaluation of sediment storage and transport for large river systems with variable flow has proven to be a challenge that often requires complex numerical models to be applied and sometimes costly direct data acquisition to be made. The present study proposes a low cost, widely available GIS-based method through which an initial assessment can be made on areas of sediment storage, transport and deposition as well as possible environmental risks that the accumulation of contaminated material may pose to riverine communities along the rivers’ channel. The method mainly relies on satellite imagery and elevation data to devise a quick model of the channel. The model has been successfully applied to study the Bolivian sector of the well-known mining-contaminated Rio Pilcomayo. The analysis shows that the portion of the channel from Villamontes to D’Orbigny accounts for more than half of the total c. 314 km2 sedimentation area of the Pilcomayo in Bolivia, and that the most environmentally problematic area is centreed around Puente Sucre, where agriculture is practiced on the contaminated floodplain. Combined with supplementary bathymetric data on the depth of the river in various points around the channel the method could offer further insight into the sediment fluxes and transport capacity of the Pilcomayo in various sectors and could thus be successfully used to assess other large mining-contaminated river channels around the world

Photo(Catalytic) Oxidation Processes for the Removal of Natural Organic Matter and Contaminants of Emerging Concern from Water

International audienc